Tottori J. Clin. Res. 9(2), 176-189, 2017
Short Neuropathology Report

Immunohistochemistry of abnormal prion protein PrPSc in an autopsy case ofsporadic type MM1 + 2 Creutzfeldt-Jakob disease (CJD)
Yoshihiro Konishi1)*, Tamami Ijiri2), Ken Watanabe3), Hirotake Nishimura4)
1) Department of Clinical Research, NHO Tottori Medical Center2) Department of Neurology, Japanese Red Cross Tottori Hospital
3) Watanabe Hospital, Meiwa-kai Medical and Welfare Center4) Department of Pathology I, Kawasaki Medical School*Correspondence: ykonishi@tottori-iryo.hosp.go.jp
AbstractWe report an autopsy case of an 80-year-old man, clinically diagnosed as a probable CJD. The clinicalcourse of the disease was 3 years and 8 months with the acute progression of dementia during the first 8 months.
The clinical characteristics were closer to those observed in type MM2 rather than type MM1. Genetic andWestern blot analyses of the abnormal prion protein (scrapie prion protein, PrPSc) confirmed this case as typeMM1 + 2. With the exception of the cerebellar lesions, the immunohistochemical characteristics of the PrPSc
were almost consistent with type MM1 + 2. In this report, we discuss particularly whether the cerebellar lesions
in the present case are characteristic of type MM1 + 2. Tottori J. Clin. Res. 9(2), 176-189, 2017
Key words: sporadic Creutzfeldt-Jakob disease (sCJD), type MM1 + 2, scrapie prion protein (PrPSc), synapticpattern, perivacuolar pattern, coarse granular deposition, plaque-like pattern, infectious CJD
Clinical course
We report an autopsy case of sporadic CJD(sCJD) in a male patient over a clinical course of3 years and 8 months with the acute progressionof dementia during the first 8 months, and deathat 80 years old.
The patient’s history revealed an operation
for retinal detachment, but no other operations,endoscopies, blood transfusions, or acupuncture
were reported. He had visited the United States
twice but not Europe. He first noticed memory
impairment at the age of 76 years. Three months
later, he visited a Neurology Clinic, where hescored 23/30 by using the revised Hasegawa
Dementia Scale (HDS-R). Thereafter, his HDS-R
score rapidly decreased to 14/30 and 3/30 at 5and 8 months after the onset, respectively. Thus,
despite a lack of ataxia, dementia progressed
rapidly during the first 8 months. The patient
experienced hallucinations, optical illusions, andemotional and behavioral abnormalities 8 months

after the onset, and visual agnosia, visuospatial
agnosia, and sensory aphasia after 9 months. He
exhibited akinetic mutism after 1 year and 3months. At 1 year and 6 months, myoclonus
began to be observed, but periodic synchronous
discharges (PSDs) were always unclear inelectroencephalo- grams (Fig. 1). However, bothdiffusion-weighted
images(DWIs)
andfluid-attenuated inversion recovery (FLAIR)
images of magnetic resonance imaging (MRI)
studies performed 3 and 9 months after the onset
had already revealed ribbon-like-shaped, highsignal intensity areas from the bilateral temporal
cortical regions to the cortices of the bilateral
occipital lobes, thus suggesting CJD (Fig. 2A). In
DWIs on MRI studies performed 2 years and 5
months after the onset, the caudate nuclei andputamen were visualized as high signal intensity
areas, in addition to the areas in the cerebral
cortices. At that time, T1-weighted images(T1WIs) on MRI studies revealed marked cortic-